1/101. bismuth encephalopathy.bismuth encephalopathy, characterized by the constant association of acute confusion, myoclonus, severe ataxia and dysarthia has reached "epidemic" proportion since its first description in 1974. The clinical aspects the pathogenic hypothesis, the diagnostic criteria as well as the report of a typical case are described by the authors, who stress the similarities with encephalopathies induced by other metals.- - - - - - - - - - ranking = 1keywords = encephalopathy (Clic here for more details about this article) |
2/101. Idiopathic pinealitis. Case report.This 63-year-old man presented with complaints of "having a feeling of falling backward" over a 3-month period. Results of his general physical examination, laboratory studies, and neurological examination were unremarkable. A magnetic resonance image revealed a 1.8 x 1.4 x 1.2-cm enhancing mass in the posterior third ventricle just above the corpora quadrigemina. The pineal gland was found to be diffusely enlarged at operation and separable from the posterior thalamus and was totally resected. The patient had an uneventful postoperative course but continues to be somewhat confused. The lesion consisted of a remarkable chronic inflammatory cell infiltrate permeating the pineal lobules and was composed of T and B lymphocytes, macrophages, eosinophils, and mast cells. Immunoperoxidase studies did not demonstrate langerhans cells, and a search for microorganisms was unrevealing. There was no evidence of neoplasia; results of immunostaining for germ cell markers and other tumor-associated antigens were negative.- - - - - - - - - - ranking = 0.16948804081824keywords = neurologic (Clic here for more details about this article) |
3/101. fluorouracil-induced neurotoxicity.OBJECTIVE: To report a case of acute neurologic adverse effects related to fluorouracil administration and to review the neurotoxicity of this agent. CASE SUMMARY: A 73-year-old white man with a history of esophageal carcinoma was treated with fluorouracil 1,500 mg iv daily for four days. After completing treatment, he presented with sudden onset of confusion, cognitive disturbances, a cerebellar syndrome, and repeated seizures. A magnetic resonance image of the brain showed no structural abnormalities, and cerebrospinal fluid examination was normal; none of the other laboratory tests provided an explanation for his symptoms. The patient was treated with anticonvulsants, and the cognitive changes resolved in 72 hours. The cerebellar signs, however, did not resolve completely and persisted when the patient was examined two weeks after discharge. DISCUSSION: fluorouracil can cause both acute and delayed neurotoxicity. Acute neurotoxicity manifests as encephalopathy or as cerebellar syndrome; seizures, as seen in our patient, have rarely been reported. Acute neurotoxicity due to fluorouracil is dose related and generally self-limiting. Various mechanisms for such toxicity have been postulated, and treatment with thiamine has been recommended. Delayed neurotoxicity has been reported when fluorouracil was given in combination with levamisole; this form of subacute multifocal leukoencephalopathy is immune mediated and responds to treatment with corticosteroids. CONCLUSIONS: Clinicians should be aware of the adverse neurologic effects of fluorouracil and should include them in the differential diagnosis when patients receiving the drug present with neurologic problems.- - - - - - - - - - ranking = 9.0347344806785keywords = neurotoxicity, encephalopathy, neurologic (Clic here for more details about this article) |
4/101. Emergency department presentations of patients with Parkinson's disease.Parkinson's disease (PD) is a chronic progressive neurological disorder characterized by tremor, muscle rigidity, slowness of movement (bradykinesia), and gait instability. In early disease, PD is well managed in an office setting, however, as the disease progresses, a variety of syndromes may result in emergency department visits. The scenarios most likely to require an emergent evaluation are severe motor "off" periods with immobility, involuntary movements (dyskinesia), psychosis, acute confusion, panic disorder, and pain. Other less frequent presentations are also discussed. This article uses illustrative cases to provide a framework to discuss emergency department diagnosis and management issues in caring for these patients.- - - - - - - - - - ranking = 0.16948804081824keywords = neurologic (Clic here for more details about this article) |
5/101. Acute confusional state as presenting feature in aneurysmal subarachnoid hemorrhage: frequency and characteristics.In many patients with subarachnoid hemorrhage (SAH) there is a delay between the onset of symptoms and admission to hospital. An important cause for the delay is an initially erroneous diagnosis. The goal of this study was to determine the frequency of acute confusional state (ACS) as a presenting symptom of SAH and to describe the clinical and radiological characteristics of these patients. We studied all 717 patients registered from January 1989 to July 1997 in the SAH database of the University Medical Center Utrecht. For patients who presented with ACS we reviewed the computed tomography scans for baseline characteristics: the amount of cisternal blood, intraventricular or intracerebral hemorrhage, and hydrocephalus. Details about features of onset were known for 646 patients. Nine patients (1.4%) presented with ACS. In five patients ACS was either preceded by a period of loss of consciousness or accompanied by severe headache. Subtle focal deficits were found at initial neurological examination in four patients. Computed tomography demonstrated a frontal hematoma in three patients and hydrocephalus in four. The site of the ruptured aneurysm was at the anterior communicating artery in four patients, at the internal carotid artery in two, and at the basilar artery in two. In our series, one per 70 patients with SAH presents with ACS. Keys to early diagnosis of SAH in patients presenting with ACS are a preceding period of loss of consciousness and severe headache on neurological assessment.- - - - - - - - - - ranking = 0.33897608163649keywords = neurologic (Clic here for more details about this article) |
6/101. Managing acutely disturbed behaviour.Managing acutely disturbed behaviour is difficult, particularly in environments which are not designed for dealing with such conduct. An understanding of factors which can lead to this behaviour and clear management policies and procedures are needed.- - - - - - - - - - ranking = 0.0012060719458358keywords = lead (Clic here for more details about this article) |
7/101. Relation of impairment to everyday competence in visual disorientation syndrome: evidence from a single case study.OBJECTIVE: To determine the relation of neurology and neuropsychology to everyday competence. DESIGN: The association of these three domains was investigated using a single case multiple baseline design with two phases. Phase A comprised 6 weeks that coincided with an inpatient admission. Phase B comprised 3 months spent at home. A battery of visual spatial tests was completed every fortnight during the A phase and at the end of the B phase. Two new tests of relevant neurologic function with control data were developed and used weekly during the A phase and at the end of the B phase. The first test recorded the speed, accuracy, and efficiency of her walking, and the second test recorded her depth perception. SETTING: Tertiary care center. PARTICIPANT: A 35-year-old woman who suffered a venous sinus thrombosis with visual disorientation syndrome. RESULTS: During Phase A, she achieved significant functional gains in mobility, dressing, bathing, and domestic tasks, in the context of unchanging psychometric test scores and static relevant neurologic function. During Phase B, she achieved few functional gains, despite improvements in neurologic status, demonstrated by depth perception. CONCLUSIONS: Everyday function can progress without improvement in neurologic and cognitive status.- - - - - - - - - - ranking = 0.67795216327297keywords = neurologic (Clic here for more details about this article) |
8/101. Hashimoto's encephalopathy.Hashimoto's encephalopathy is a subacute condition associated with autoimmune thyroiditis. Its presentation varies from focal neurologic deficits to global confusion. Unlike encephalopathy associated with hypothyroidism, Hashimoto's encephalopathy responds to steroid therapy and not thyroxine replacement.- - - - - - - - - - ranking = 1.5694880408182keywords = encephalopathy, neurologic (Clic here for more details about this article) |
9/101. Novel prion protein gene mutation in an octogenarian with Creutzfeldt-Jakob disease.BACKGROUND: The transmissible spongiform encephalopathies constitute a fascinating and biologically unique group of invariably fatal neurodegenerative disorders that affect both animals and humans. Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker syndrome, and fatal familial insomnia represent the more common human phenotypes. Excluding the small number of iatrogenically transmitted cases, approximately 85% to 90% of patients develop CJD without identifiable explanation, with an increasing number of different mutations in the prion protein gene (PRNP) recognized as probably causative in the remainder. OBJECTIVE: To report on an 82-year-old woman with pathologically confirmed CJD found unexpectedly to harbor a novel mutation in PRNP. methods: Routine clinical investigations were undertaken to elucidate the cause of the rapidly progressive dementia and neurological decline manifested by the patient, including magnetic resonance imaging of the brain, electroencephalography, and cerebrospinal fluid analysis for the 14-3-3 beta protein. Standard postmortem neuropathological examination of the brain was performed, including immunocytochemistry of representative sections to detect the prion protein. Posthumous genetic analysis of the open reading frame of PRNP was performed on frozen brain tissue using polymerase chain reaction and direct sequencing. RESULTS: Concomitant with the exclusion of alternative diagnoses, the presence of characteristic periodic sharp-wave complexes on the electroencephalogram in combination with a positive result for 14-3-3 beta protein in the cerebrospinal fluid led to a confident clinical diagnosis of CJD, confirmed at autopsy. There was no family history of dementia or similar neurological illness, but patrilineal medical information was incomplete. Unexpectedly, full sequencing of the PRNP open reading frame revealed a single novel mutation consisting of an adenine-to-guanine substitution at nucleotide 611, causing alanine to replace threonine at codon 188. CONCLUSIONS: In addition to expanding the range of PRNP mutations associated with human prion diseases, we believe this case is important for the following reasons. First, from an epidemiological perspective, the avoidance of occasional incorrect classification of patients manifesting neurodegenerative disorders that may have a genetic basis requires systematic genotyping, particularly when there are uncertainties regarding the family history. Second, the incidence of spongiform encephalopathy in elderly patients beyond the typical age range may be underestimated and does not preclude a genetic basis. Finally, as a corollary, this case highlights problematic issues in human transmissible spongiform encephalopathies, as illustrated by disease penetrance and age of onset in genotype-phenotype correlations.- - - - - - - - - - ranking = 0.53897608163649keywords = encephalopathy, neurologic (Clic here for more details about this article) |
10/101. Acute encephalopathy associated with metronidazole therapy.A forty-eight year-old male with amoebic liver abscess became encephalopathic 3 days following oral metronidazole. Withdrawal of the drug led to prompt resolution of all encephalopathic symptoms.- - - - - - - - - - ranking = 0.8keywords = encephalopathy (Clic here for more details about this article) |
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